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Atkins or Ammonia?

Nitrogen Metabolism and the Urea Cycle

By Stephanie Dingwall, Tammy Nguyen

Atkins or Ammonia?


 

Abstract

This interrupted case study is an exploration of ornithine transcarbamylase (OTC) deficiency, which results from a genetic defect in a urea cycle enzyme. Students are introduced to a 21-year-old patient who loses consciousness after a gym session. Upon investigation of the man’s signs and symptoms, students apply their knowledge of nitrogen metabolism, the urea cycle, and how the latter is linked to the Krebs cycle to diagnose and treat the patient. Before beginning the case, students are expected to have some basic knowledge of amino acids, general metabolism, and genetics, as it is designed for an upper-level undergraduate course in biochemistry. The case incorporates multiple levels of analysis, from simple recall to synthesizing and applying information. The case can be used for either in-person or online course offerings, and can be implemented via group work or individually. Part of the case can also be assigned prior to class, or it can be fully completed within class time, ending with an optional discussion of OTC deficiency.

   

Date Posted

08/01/2022

Overview

Objectives

  • Predict products and substrates resulting from a urea cycle disorder (UCD), given appropriate clinical data.
  • Draw and review the urea cycle after it is clear the reason for the abnormal clinical data is a urea cycle disorder (UCD).
  • Make connections between different metabolic reactions and pathways, e.g., the urea cycle link with the Krebs cycle, phosphate synthetase I (CPS I) with the urea cycle, and carbamoyl phosphate synthetase II (CPS II) with pyrimidine biosynthesis.

Keywords

Urea cycle; ornithine transcarbamylase deficiency; OTC deficiency; genetics; nitrogen metabolism; hyperammonemia; nitrogen metabolism; Krebs cycle; urea cycle disorder; UCD

  

Subject Headings

Biochemistry
Genetics / Heredity
Physiology

EDUCATIONAL LEVEL

Undergraduate upper division

  

FORMAT

PDF

   

TOPICAL AREAS

N/A

   

LANGUAGE

English

   

TYPE/METHODS

Interrupted

 

 

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